![Most people with ALS (amyotrophic lateral sclerosis, Lou Gehrig's disease) die within three to five years of the first symptoms from respiratory failure. About 10% of patients survive for 10 or more years.](https://images.medicinenet.com/images/article/main_image/brain-tumor-cancer-cell-disease-microscopic.jpg?output-quality=75)
ALS (amyotrophic lateral sclerosis, also called Lou Gehrig's disease) is a rare and fatal neurological motor neuron disease that damages the nerves that control muscles, causing muscle weakness. The muscles gradually weaken over time, start to twitch (fasciculations), and waste away (atrophy) and the brain loses the ability to control voluntary movements.
Most people with ALS die within three to five years of symptom onset from respiratory failure but about 10% of patients survive for 10 or more years.
What Are Symptoms of ALS?
Symptoms of ALS (amyotrophic lateral sclerosis) include:
- Weak arm or leg muscles, usually starting in one arm or leg, then spreading to other parts of the body
- Weak muscles in the hands or arms can lead to problems with:
- Using buttons or zippers
- Writing
- Handling coins and other small objects
- Weak muscles in the legs or feet can lead to problems with:
- Walking and turning
- Standing up
- Tripping or falling
- Balance
- Climbing stairs
- Feeling as if the legs are “heavy”
- Dragging the foot on the ground when walking
- Weak muscles in the hands or arms can lead to problems with:
- Weak muscles in the head, neck, or chest, can lead to problems with:
- Difficulty moving the mouth and speaking clearly
- Trouble chewing and swallowing
- Stiff jaw and difficulty opening the mouth
- Drooling
- Inability to fully close the eyes
- Trouble holding up the head due to weak neck muscles
- Trouble breathing
- Slow, stiff, or awkward movements
- Muscle twitches and spasms
- Mood changes such as laughing, crying, or yawning at times when it doesn't make sense
- Problems with thinking and language
- Anxiety or depression due to loss of functioning
- A small percentage may develop dementia over time
What Causes ALS?
About five to 10 percent of cases of ALS (amyotrophic lateral sclerosis) occur in people who have a family history of ALS, and the cause is believed to be genetic in those cases.
The other 90 to 95 percent of ALS occurs sporadically, with no family history of the disease. It is believed there are genetic variations that can make a person more susceptible to sporadic ALS.
Risk factors for developing sporadic ALS include:
- Race/ethnicity
- Caucasians have higher rates of ALS than other races
- Gender
- Prior to the age of 65-70, the incidence of ALS is higher in men than in women, but after that the incidence is equal
Possible causes of sporadic ALS include:
- Oxidative stress
- Mitochondrial dysfunction
- Immune system abnormalities
- Glutamate toxicity
- Toxic exposures
- The incidence of ALS cases among Gulf War veterans is two times higher than expected, possibly due to cyanobacteria that live in desert sands
- Higher-than-average rates of ALS on the island of Guam may be due to native peoples' diet of poisonous nuts from indigenous cycad trees
How Is ALS Diagnosed?
ALS (amyotrophic lateral sclerosis) is diagnosed with a patient history and physical examination. Tests used to help confirm a diagnosis of ALS include:
- Electromyography (EMG), to check how well nerves and muscles are working
- Magnetic resonance imaging (MRI) of the brain and spinal cord
- Blood and urine tests
- Tests to rule out infectious diseases such as:
- Human immunodeficiency virus (HIV)
- Human T-cell leukemia virus (HTLV)
- Polio
- West Nile virus
- Tests to rule out neurological disorders such as:
- Multiple sclerosis
- Post-polio syndrome
- Multifocal motor neuropathy
- Spinal and bulbar muscular atrophy (Kennedy’s disease)
What Is the Treatment for ALS?
There is no cure for ALS (amyotrophic lateral sclerosis). Treatment for ALS includes medications and supportive care for symptoms.
- Medications
- Edaravone (Radicava)
- May help slow the progression of the disease in some patients
- Medicines to treat symptoms
- Muscle spasms and weakness
- Drooling
- Sleep problems
- Pain
- Depression
- Other treatments to cope with the symptoms of ALS
- Nutritional support from a nutritionist/dietitian
- Feeding tube
- Assistive devices to help with walking
- Cane
- Crutches
- Motorized wheelchair
- Assistive devices to help with talking, such as a special computer that patients control with hand or eye movements
- Physical therapy
- Speech therapy
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https://www.uptodate.com/contents/amyotrophic-lateral-sclerosis-als-the-basics?search=ALS&source=search_result&selectedTitle=1~150&usage_type=default&display_rank=1
https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Amyotrophic-Lateral-Sclerosis-ALS-Fact-Sheet
https://www.mda.org/disease/amyotrophic-lateral-sclerosis/causes-inheritance